Disease Associations

Intracranial Aneurysm

The most serious possible complication of PKD is a cerebral or brain aneurysm (a bulging blood vessel due to weakening of the blood vessel wall). Aneurysms can rupture, causing bleeding into the brain (subarachnoid hemorrhage). If not treated before the aneurysm ruptures, this can lead to irreversible brain damage or death. Aneurysm rupture occurs most often in people with larger aneurysms (>10 mm). The most common symptom of subarachnoid hemorrhage is a severe headache that begins suddenly, frequently with nausea and vomiting.

Approximately 3 to 7 percent of young adults with ADPKD may have brain aneurysms, and the frequency increases to 12 to 15 percent if someone else in the patient’s family has had an intracranial aneurysm. Compared with the general population, the risk of developing an aneurysm in ADPKD is approximately fivefold greater. People with a first-degree relative with a history of intracranial aneurysm or subarachnoid hemorrhage are at the highest risk of forming an aneurysm.

Early detection (before symptoms occur) of intracranial aneurysms is recommended in people who are at high risk. Screening (looking for an aneurysm before it ruptures) is generally performed with magnetic resonance angiography (MRA) or, if not available, a computed tomography (CT) scan.

Routine screening is recommended only for high-risk patients, such as those with a previous rupture, a positive family history of a brain hemorrhage or stroke, warning symptoms (eg, unusual headaches), in whom a loss of consciousness would place the patient or others at extreme risk.

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